Article
The Mitochondrial m-AAA Protease Prevents Demyelination and Hair Greying.
PLoS genetics - 1 Dec 2016
Wang Shuaiyu, Jacquemyn Julie, Murru Sara, Martinelli Paola, Barth Esther, Langer Thomas, Niessen Carien M, Rugarli Elena I
Abstract excerpt
The m-AAA protease preserves proteostasis of the inner mitochondrial membrane. It ensures a functional respiratory chain, by controlling the turnover of respiratory complex subunits and allowing mitochondrial translation, but other functions in mitochondria are conceivable. Mutations in genes encoding subunits of the m-AAA protease have been linked to various neurodegenerative diseases in humans, such as...
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